Disease Information for Hereditary autonomic/Sensory neuropathy (HSN III)

Clinical Manifestations
Signs & Symptoms
Chronic Diarrhea in a Child
Anesthetic hypesthetic limbs/areas
Autonomic instability/Variable BP/HR
Loss of pain of temperature sensation
Loss pain/temperature sense/light touch preserved
Mental Deficiency Child
Sensory defects/lower extremities
Symmetric Polyneuropathy
Demographics & Risk Factors
Family History
Family history/Mental retardation
Associated Diseases & Rule outs
Rule Outs
Diabetic autonomic neuropathy syndrome
Associated Disease & Complications
Autonomic neuropathy
Charcot neuroarthropathic joint
Hereditary sensory radicular neuropathy
Mental retardation
Orthostatic hypotension syndrome
Pain, congenital indifference
Painful neuropathy
Peripheral neuropathy
Sensory neuropathy
Disease Mechanism & Classification
CLASS/Neurologic (category)
CLASS/Peripheral nerve disease (ex)
Pathophysiology/Gene locus Chromosome 1
Pathophysiology/Delayed gastric emptying/gastrostasis
Pathophysiology/Hereditary Neuropathy
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Hereditofamilial (category)
PROCESS/Mononeuropathy tendency (ex)

Neuropathy, Hereditary Sensory, Type IV; Familial Dysautonomia, Type II; HSAN IV; HSN IV; Hereditary Sensory and Autonomic Neuropathy IV; Insensitivity to Pain, Congenital, with Anhydrosis; CIPA; Neuropathy, Congenital Sensory, with Anhydrosis; The hereditary sensory neuropathies (HSN) include 4-6 similar but distinct inherited degenerative disorders of the nervous system (neurodegenerative) that frequently progress to loss of feeling, especially in the hands and feet; The classification of these diseases is complicated, and sometimes a source of disagreement among the experts; Hereditary sensory neuropathy type IV (HSN4) is a rare genetic disorder characterized by the loss of sensation (sensory loss), especially in the feet and legs and, less severely, in the hands and forearms; The sensory loss is due to abnormal functioning of small, unmyelinated nerve fibers and portions of the spinal cord that control responses to pain and temperature as well as other involuntary or automatic body processes; Sweating is almost completely absent with this disorder; Mental retardation is usually present; The disorder is inherited as an autosomal recessive trait; The gene involved is located on chromosome 1; HSNs of various types may attack a single nerve (mononeuropathy) or many nerves simultaneously (polyneuropathy); The resulting symptoms may involve sensory, motor, reflex, or blood vessel (vasomotor) functions----[ NORD]---------


External Links Related to Hereditary autonomic/Sensory neuropathy (HSN III)
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)